Neurologic phenomenon in which one or less commonly
both pupils are dilated and respond either slowly or not at all to light. It
can be associated with autonomic nervous system instability (syncope, vagal
hyporeflexia, postural hypotension), slow gastric emptying, and tendon
Holmes Adie Syndrome; Constitutional Areflexy-Tridoplegia
Interna; Myotonic Pupil; Myotonic Pupillary Reaction; Pseudo
Argyll-Robertson Syndrome; Weill-Reys Syndrome; Saenger Syndrome.
Described in 1931 by William John Adie, a British
physician and neurologist. However, the disease was mentioned earlier by
many others. John Hughlings Jackson described the disease at the end of the
19th century. Max Nonne, J. Strassberger, and Alfred Saenger described it
just at the beginning of the 20th century. Georges Weill and L. Reys
probably were the first to describe this disease as its own (nonsyphilitic)
The features of Adie syndrome are idiopathic but
also may appear secondary to other disorders (e.g., diabetes, syphilis). The
pupils are sensitive to methacholine (quaternary ammonium
The following signs are crucial for diagnosis: either
unilateral or bilateral pupillotonia (tonic, sluggish reaction of pupil[s]
to light) with light-near dissociation of accommodation (condition in which
the pupillary reflex response to light is either absent or abnormal, while
the near response is still intact) and hypoactive or absent tendon reflexes.
Evidence indicates Adie syndrome is actually the
first sign of a gradually progressive, usually benign, autonomic
dysfunction. Therefore, other signs of autonomic dysfunction must be sought.
History should elicit symptoms of
syncope, vagal hyporeflexia, postural hypotension, sweating abnormalities
with patchy anhidrosis, bladder and bowel dysfunction, erectile and
ejaculatory dysfunction, and delayed gastric emptying. Signs to elicit in
addition to those pertaining to the eye may include blood pressure
measurements in the lying and standing position (Schellong test).
Investigations should include ECG, possibly 24-hour ECG, and results of
carotid sinus massage or tilt-table provocation test for arrhythmia.
In patients with considerable autonomic
dysfunction, profound bradycardia may occur in response to painful stimuli.
Precautions (rapid sequence induction) must be taken if the patient is
suspected of having a full stomach. Direct laryngoscopy may produce an
unexpectedly large swing in arterial blood pressure, and this response may
usefully be obtunded by adequate depth of anesthesia.
Atropine or other anticholinergic
drugs may be useful agents in patients prone to bradyarrhythmia.
Bacon PJ, Smith SE: Cardiovascular and sweating dysfunction in patients
with Holmes-Adie syndrome. J Neurol Neurosurg Psychiatry